Acute life-threatening presentation of unknown lymphatic malformation.

Lymphatic malformations (LMs) are rare congenital vascular malformations. Lymphatic malformations include a wide variety of diseases, such as lymphangiomatosis, macro or microcystic lymphatic malformation, and lymphangiectasia. Lymphatic malformations are often first seen at birth, although intrauterine diagnosis is not uncommon. Depending on their size and location, they can be asymptomatic or threaten life by compression of vital structures. We report 2 children admitted to the emergency department with an acute life-threatening presentation of undiagnosed LM. They both required immediate cardiopulmonary resuscitation. Because acute presentations of LM are rare, proper diagnosis can be difficult. Emergency physicians must be aware that sudden enlargement of an unknown thoracic LM can result in airway compression, cardiac tamponade, or rapidly progressive pleural or pericardial effusion. Prompt diagnosis is essential to avoid further morbidity or mortality. The treatment of extensive forms is challenging, requiring a multidisciplinary approach. The prognosis can be poor. Lymphaticmalformations (LMs) consist of rare andcomplex congenital disorders of the development of the lymphatic system. Several forms of LMs are described depending on the timing of the arrest in lymphangiogenesis, including micro and macrocystic lymphatic malformation, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasias [1,2]. Although most of LMs are diagnosed in utero or during the first 2 years of age, it can be diagnosed as late as in adulthood. Diagnosis requires recognition of the clinical features, including bone and spleen involvement, analysis of fluid effusion, and imaging studies. The evolution is usually slow, but rapid deterioration can occur after local infection or bleeding into the malformation or accumulation of chyle in a serosal cavity. A previously healthy 22-month-old boy presented in the emergency department with rapidly progressive respiratory distress associated with a 24-hour history of rhinitis, without fever.Onadmission,hepresentedrespiratory insufficiencywith obnubilation, exhaustion, and hypoxemia despite full flow face mask oxygen. On auscultation, air entry was very poor. There 0735-6757/$ – see front matter © 2010 Elsevier Inc. All rights reserved. was no evidence of neck swelling. The child required urgent endotracheal intubation, but airway stabilization was complicated by a severe upper airways deviation. In the following hours, a suprasternal and left laterocervical swelling appeared. A magnetic resonance imaging (Fig. 1) revealed a macrocystic lymphatic malformation involving the neck and the upper mediastinum. The child underwent a complete surgical resection with favorable outcome, except for vocal cord paralysis. A 16-month-old boy was admitted to the emergency department for cardiorespiratory collapse. This child had been previously observed for several episodes of respiratory distress for which the diagnosis of asthma was suggested. Previous investigations, including a chest x-ray, were normal. On admission, the child was in shock; the heart rate was 200 beats per minute, respiratory rate was 40 beats per minute, blood pressure was 75/35 mm Hg, and oxygen saturation as measured by pulse oximetry was 93% on full face mask oxygen. The auscultation was characterized by diffuse and bilateral crepitations. An enlarged spleen was palpable at abdominal examination. Chest x-ray revealed severe